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Pediatr Radiol

DOI 10.1007/s00247-014-2896-1

CASE REPORT

Duodenal atresia: not always a double bubble


Jonathan M. Latzman & Terry L. Levin &
Suhas M. Nafday

Received: 9 October 2013 / Revised: 29 December 2013 / Accepted: 22 January 2014


# Springer-Verlag Berlin Heidelberg 2014

Abstract A newborn infant with a prenatal diagnosis of [3]. Radiographs in infants with duodenal atresia rarely dem-
duodenal atresia and abdominal radiographs demonstrating onstrate air in distal bowel due to the anomalous development
air in distal bowel is presented. An upper gastrointestinal of the biliary tree that serves as a conduit between the
series revealed complete duodenal obstruction and duodenal obstructed proximal duodenum and the non-obstructed distal
atresia was confirmed at surgery. The significance of distal duodenum. In these infants the diagnosis of bowel atresia may
bowel gas and the embryological development of this unusual be missed. We present a case and discuss the embryological
entity is discussed. development of this unusual entity.

Keywords Duodenum . Radiography . Upper


Gastrointestinal series . Duodenal atresia . Anomalous duct . Case report
Infant
A 32 weeks’ preterm male infant with a prenatal diagnosis of
duodenal atresia and polyhydramnios was admitted to the
neonatal intensive care unit following a cesarean section for
Introduction
complete breech presentation. The infant was apneic and
cyanotic at birth and initially required positive pressure ven-
Duodenal atresia, a failure of recanalization of the duodenum
tilation. An abdominal radiograph was obtained and demon-
during embryonic life, occurs with a frequency of 1 in 7,500
strated an air-filled, distended stomach and proximal duode-
births [1]. Antenatal sonographic findings include
num with air distal to the duodenum (Fig. 1).
polyhydramnios as well as dilatation of the stomach and
An upper gastrointestinal series (UGI) revealed complete
proximal duodenum [2]. An infant with duodenal atresia
obstruction to the flow of contrast at the second portion of the
typically presents in the first few hours of life with bilious
duodenum (Fig. 1). A US demonstrated a fluid-filled proximal
emesis. (The emesis may be nonbilious if the atresia occurs
duodenum and intermittent foci of air within the pancreatic
above the ampulla.) Classically, the abdominal radiograph
duct (Fig. 1). The superior mesenteric artery and vein were
demonstrates the “double bubble,” representing the air-filled
normally positioned.
stomach and duodenum. Distal bowel gas is typically absent
On day two of life, the patient underwent an uncomplicated
duodenoduodenostomy for duodenal atresia. Malrotation was
J. M. Latzman : T. L. Levin
identified and a Ladd procedure and appendectomy were
Department of Radiology, Division of Pediatric Radiology, performed. There was no annular pancreas. The infant’s post-
Jack D. Weiler Hospital, operative course was uneventful and he was discharged home
Children’s Hospital at Montefiore-Albert Einstein College of Medicine, at 39 days of age.
Bronx, NY, USA

S. M. Nafday (*)
Department of Pediatrics, Division of Neonatology, Discussion
Jack D. Weiler Hospital,
Children’s Hospital at Montefiore-Albert Einstein College of Medicine,
1825 Eastchester Road, Bronx, NY 10461, USA During the 5th to 10th weeks of gestation, the duodenal lumen
e-mail: snafday@montefiore.org is occluded by rapidly proliferating epithelial cells. It later
Pediatr Radiol

Fig. 1 Premature infant with


prenatal diagnosis of duodenal
atresia and polyhydramnios. a
Abdominal radiograph
demonstrates an air-filled
distended stomach and proximal
duodenum with air distal to the
duodenum. b Image from an UGI
reveals complete obstruction to
the flow of contrast at the second
portion of the duodenum. c, d
Transverse sonographic images of
the pancreatic head demonstrate a
fluid-filled proximal duodenum
and intermittent foci of air (arrow)
within the pancreatic duct
(arrowhead). duod duodenum,
panc pancreas

recanalizes from cephalad to caudad by a process of coales- enter a short segment of the duodenum. When the two duo-
cence of vacuoles that develop within the solid cord of the denal channels fuse, one hepatopancreatic duct and one ac-
duodenum. The second portion of the duodenum is cessory pancreatic duct typically regress [4].
recanalized last. Initially, the duodenum is recanalized into In duodenal atresia, the second portion of the duodenum
two parallel channels, each receiving its own hepatopancreatic fails to undergo recanalization, which results in proximal
duct and accessory pancreatic duct. In total, four ducts initially duodenal obstruction. Rarely, as in our case, duodenal atresia
Pediatr Radiol

Fig. 2 Full-term infant with a


prenatal history of duodenal
atresia and distal bowel gas. a
Radiograph obtained at 6 h of life.
b Lateral oblique image from a
UGI series demonstrates
obstruction to the flow of contrast
at the level of the second portion
of the duodenum. There is filling
of a linear channel below the
duodenal bulb that may represent
an anomalous duct (arrow). No
annular pancreas was present at
surgery

is associated with persistence of the primitive hepatic and duodenum is anastomosed to the distal duodenum. At surgery,
pancreatic ducts. Multiple configurations of bifucating anom- dissection of the biliary anatomy is avoided to prevent ductal
alous ducts, with limbs entering proximal and distal to the injury [7] . Bile flow into the duodenum is observed intraop-
atretic portion of duodenum, have been reported [4, 5]. The eratively by gentle compression of the gallbladder to identify
temporal and anatomical proximity of the developing biliary the ampulla of Vater. When dual bile ducts are encountered,
system to the duodenum may account for the occurrence of both ampullae are identified to prevent injury. In some cases,
this abnormality. The developing hepatopancreatic and acces- an intraoperative cholangiogram may be performed to docu-
sory pancreatic ducts may interfere with normal duodenal ment patency of both biliary ampullae [8].
vacuolization and recanalization. Alternatively, abnormal The true extent of bifurcating anomalous ducts is likely
duodenal recanalization may result in anomalous duct for- underreported. In a report of 44 infants with duodenal atresia
mation. Of interest, the site of atresia occurs at the level of and no distal bowel gas on plain abdominal radiographs,
the ampulla of Vater in approximately 83% of cases of three were found to have anomalous bifurcated bile duct
duodenal atresia [6]. Additionally, while biliary atresia is termination discovered intraoperatively by compression of
uncommon in infants with duodenal atresia, biliary stenosis the gallbladder [5].
[1, 2, 7] as well as pancreatic ductal atresia [1] have been In summary, the presence of distal bowel gas in an infant
described. does not exclude the diagnosis of duodenal atresia. While
In infants with duodenal atresia and anomalous biliary other entities resulting in partial duodenal obstruction are
ducts that traverse the site of atresia, air may bypass the atresia more common and may be potentially more dangerous (midgut
through the anomalous ducts and the plain radiograph may volvulus), in the correct clinical setting, duodenal atresia with
demonstrate distal bowel gas; the classic double bubble anomalous biliary ductal anatomy should be considered. Pre-
will be absent. Additionally, the duodenal bulb may only natal sonographic findings suggestive of duodenal atresia
be mildly distended with air or not distended at all. should not be dismissed based on the plain film findings.
Prenatal imaging may suggest the true diagnosis; however, UGI series is diagnostic.
based on the plain radiograph, an erroneous diagnosis of a
partial duodenal obstruction due to annular pancreas, Ladd
bands or duodenal web may be made. UGI series is Conflicts of interest None
diagnostic and will demonstrate obstruction to the flow
of contrast beyond the second portion of the duodenum.
The anomalous duct may be filled with contrast (Fig. 2). References
Sonography may be helpful in some instances; sonography
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Pediatr Radiol

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